Pediatric Hematology and Oncology

Match each pediatric patient's clinical and laboratory presentation to the most likely hematologic or oncologic diagnosis.

A $3$-year-old boy is brought to the pediatrician's office by his father, who noticed a firm, painless swelling on the right side of the child's abdomen while dressing him. The child has been eating well, is as active as usual, and has had no fever, vomiting, or bone pain. Vital signs are within normal limits for age, except for a blood pressure of $120/78$ mmHg (which is $>95$ th percentile for his age). On physical examination, a smooth, firm, non-tender mass is palpated in the right upper and lower quadrants; it does not cross the midline. The remainder of the examination is unremarkable. Urinalysis reveals $10$-$15$ red blood cells per high-power field. Which of the following is the most appropriate next step in the evaluation of this patient?

An $8$-year-old boy with a known history of severe hemophilia A is brought to the emergency department by his parents after falling from a tree and striking his head. The parents report a brief loss of consciousness. He is now awake but complains of a severe headache and has vomited twice. His vital signs are temperature $37.1^\circ\text{C}$, heart rate $110\text{ /min}$, blood pressure $115/75\text{ mmHg}$, and respiratory rate $20\text{ /min}$. Physical examination reveals a large boggy hematoma over the right parietal region. Neurologic examination shows a Glasgow Coma Scale score of $14$ (confused conversation). Arrange the following management steps in the most appropriate chronological order for this patient.

The parents of a 6-month-old boy of Mediterranean descent bring him to the clinic for a routine evaluation. They report that he has been increasingly fussy, easily fatigued, and feeding poorly over the past month. The parents also express two additional concerns: they are worried because he cannot sit up independently, and they request he receive his measles, mumps, and rubella (MMR) vaccine today to finish his immunizations early. He was born at full term, and his growth has consistently tracked along the 50th percentile. His temperature is $37.0^\circ\text{C}$ ($98.6^\circ\text{F}$), heart rate is $155/\text{min}$, and respiratory rate is $36/\text{min}$. On physical examination, the infant appears notably pale. A grade 2/6 systolic ejection murmur is auscultated at the left lower sternal border. The liver edge is palpated $3\text{ cm}$ below the right costal margin, and the spleen is palpable $4\text{ cm}$ below the left costal margin. Neurologic examination reveals normal symmetric tone; he sits with truncal support and successfully rolls from front to back.

Laboratory testing reveals:
Hemoglobin: $6.2\text{ g/dL}$
Mean corpuscular volume (MCV): $62\text{ fL}$
Reticulocyte count: $7\%$
Thyroid-stimulating hormone (TSH): $2.5\text{ \mu U/mL}$ (normal: $0.5-5.0\text{ \mu U/mL}$)
Free T4: $1.4\text{ ng/dL}$ (normal: $0.9-2.3\text{ ng/dL}$)

A peripheral blood smear shows microcytic, hypochromic red blood cells with numerous target cells.

Which of the following is the most appropriate next step in the management of this patient?

Evaluate the following histologic, cytogenetic, and clinical descriptions. Pair each specific finding with its corresponding pediatric hematologic or oncologic diagnosis.

During a routine well-child visit, the mother of a $4$-year-old girl expresses concern about multiple new bruises on her daughter's legs over the past few days. The mother notes the child had a self-resolving upper respiratory tract infection approximately three weeks prior. The patient is otherwise active and has no history of excessive bleeding, epistaxis, or melena. Physical examination reveals a well-appearing, playful child. Vital signs are within normal limits for age. Skin examination shows scattered petechiae and small ecchymoses confined to the bilateral lower extremities. There is no jaundice, lymphadenopathy, or hepatosplenomegaly.

Laboratory evaluation demonstrates:
Hemoglobin: $12.8 \text{ g/dL}$
Leukocyte count: $8,200/\text{mm}^3$ (normal differential)
Platelet count: $16,000/\text{mm}^3$

Which of the following is the most appropriate next step in management for this patient?

A $6$-year-old boy is brought to the clinic by his parents due to fatigue and yellowish discoloration of his eyes for the past $3$ days. He had a mild upper respiratory tract infection one week ago. His past medical history is unremarkable, and his immunizations are up to date. Family history is notable for a paternal aunt who underwent an unexplained splenectomy in her twenties. Vital signs are within normal limits. Physical examination reveals scleral icterus, pale conjunctivae, and a palpable spleen edge $4 \text{ cm}$ below the left costal margin. Laboratory studies show the following:

Direct antiglobulin (Coombs) testing is negative.

Which of the following is the most appropriate next step in diagnostic evaluation?

A $9$-year-old boy is diagnosed with Burkitt lymphoma after presenting with a rapidly enlarging abdominal mass and abdominal pain. Laboratory studies reveal a white blood cell count of $14,000/\text{mm}^3$, a uric acid level of $11.2 \text{ mg/dL}$, potassium of $5.1 \text{ mEq/L}$, phosphorus of $6.5 \text{ mg/dL}$, and calcium of $7.2 \text{ mg/dL}$. His serum creatinine is $0.8 \text{ mg/dL}$. He is admitted to the pediatric intensive care unit for management. Arrange the following steps in the most appropriate chronological order for the prevention and management of tumor lysis syndrome (TLS) and its potential complications in this patient, starting from the initial intervention upon admission.

Match each pediatric patient's clinical and laboratory presentation to the most likely underlying diagnosis and its associated pathophysiologic mechanism.

A $2$-year-old boy with sickle cell disease (HbSS) is brought to the emergency department by his parents due to profound weakness and a rapidly enlarging abdomen over the past $6$ hours. He has been unusually sleepy and difficult to arouse. His temperature is $37.0^\circ\text{C}$ ($98.6^\circ\text{F}$), heart rate is $168/\text{min}$, blood pressure is $70/40\text{ mm Hg}$, and respiratory rate is $32/\text{min}$. Physical examination reveals marked conjunctival pallor. His abdomen is distended, firm, and diffusely tender. The spleen edge is palpable $6\text{ cm}$ below the left costal margin, which is a new finding compared to his baseline. Capillary refill time is $4$ seconds. Laboratory studies reveal:

Hemoglobin: $4.2\text{ g/dL}$ (baseline $8.5\text{ g/dL}$)
Reticulocytes: $14\%$
Platelets: $75,000/\text{mm}^3$
Leukocytes: $12,500/\text{mm}^3$

Which of the following is the most appropriate next step in management?

A $6$-year-old boy is brought to the emergency department by his parents due to a new-onset fever and a mild cough that started earlier today. The patient was diagnosed with acute lymphoblastic leukemia (ALL) $4$ months ago and is currently receiving maintenance chemotherapy. His last chemotherapy cycle was $10$ days ago. His temperature is $39.4^\circ\text{C}$ ($102.9^\circ\text{F}$), pulse is $125/\text{min}$, respirations are $24/\text{min}$, and blood pressure is $95/60 \text{ mmHg}$. On physical examination, the child appears pale and mildly lethargic but is easily rousable. Lung auscultation is clear bilaterally without wheezing or crackles. There is a central venous port in the right upper chest with no surrounding erythema or induration.

Laboratory studies show:
Leukocyte count: $800/\mu\text{L}$
- Neutrophils: $15\%$
- Lymphocytes: $80\%$
- Monocytes: $5\%$
Hemoglobin: $9.2 \text{ g/dL}$
Platelet count: $85,000/\mu\text{L}$

Which of the following is the most appropriate next step in the management of this patient?

A pediatric oncology team is reviewing pathology, laboratory, and imaging reports for several newly admitted patients. Match each patient's characteristic diagnostic profile to the most accurate underlying solid tumor diagnosis.

An adolescent girl, aged $10$, is evaluated in the outpatient hematology clinic due to recurrent epistaxis and progressive fatigue over the past three months. Her adoptive parents report she has always been significantly smaller than her peers. Vital signs are within normal limits for her age. Physical examination reveals short stature (below the $3$ rd percentile), microcephaly, an absent right thumb, and several flat, hyperpigmented macules across her abdomen and back. Laboratory testing shows a hemoglobin of $7.2 \text{ g/dL}$, a mean corpuscular volume of $112 \text{ fL}$, a leukocyte count of $1,800/\text{mm}^3$, and a platelet count of $22,000/\text{mm}^3$. Which of the following is the most likely underlying mechanism for this patient's clinical and laboratory findings?

A 5-year-old boy with a known history of severe hemophilia A is brought to the pediatric emergency department after falling from a playground structure and striking his head. His parents report that he has vomited twice since the fall and appears unusually lethargic. On primary survey, his airway is patent, breathing is unlabored, and perfusion is adequate. His Glasgow Coma Scale score is 13. Arrange the following management steps in the most appropriate chronological order.

A 24-month-old toddler is brought to the pediatric clinic by her parents, who noticed an unusual lump in her abdomen while dressing her. Over the past month, the child has been increasingly fussy, has experienced intermittent bone pain, and has lost a noticeable amount of weight. Vital signs show a temperature of 37.2°C (99.0°F), a heart rate of 115/min, and a blood pressure of 138/88 mmHg. Physical examination reveals a firm, irregular, nodular abdominal mass that extends across the anatomic midline. Additional findings include bilateral periorbital ecchymoses and a right-sided mild ptosis with miosis. Which of the following laboratory studies is most appropriate to help confirm this patient's most likely diagnosis?

Parents bring their $3$-year-old son to the clinic after noticing he has been uncharacteristically lethargic and refusing to walk for the past two weeks. The child has also experienced intermittent subjective fevers. Vital signs show a temperature of $38.2^\circ\text{C}$ ($100.8^\circ\text{F}$), heart rate of $115$/min, blood pressure of $92/60$ mmHg, and respiratory rate of $22$/min. On examination, the patient appears pale and tired. There are widespread petechiae on his shins and forearms. Palpation of the abdomen reveals the liver edge $4$ cm below the right costal margin and the spleen tip $5$ cm below the left costal margin. Cervical lymph nodes are enlarged, firm, and non-tender. The physician suspects a primary hematologic malignancy and initiates a comprehensive evaluation. Arrange the following diagnostic and staging steps in the most appropriate chronological sequence.

A 5-year-old girl with acute lymphoblastic leukemia (ALL) comes to the hematology-oncology clinic with her father for a routine visit. She is currently in the maintenance phase of her treatment regimen, taking daily oral mercaptopurine and weekly methotrexate. Her father reports that she developed a mild cough and runny nose two days ago but has remained afebrile and active. He also expresses concern that she is unable to ride a two-wheeled bicycle without training wheels, although she effortlessly pedals her tricycle.

Vital signs show a temperature of $36.9^\circ\text{C}$ ($98.4^\circ\text{F}$), a heart rate of $98/\text{min}$, a blood pressure of $95/60\text{ mmHg}$, and a respiratory rate of $18/\text{min}$. Physical examination reveals clear rhinorrhea and clear lung fields bilaterally.

The father notes that she is due for her kindergarten immunizations, having last received vaccines at $18$ months of age.

Which of the following is the most appropriate next step in the management of this patient?

A medical student is reviewing pathologic specimens during a pediatric hematology and oncology rotation. Match each described histologic or peripheral blood smear finding with the most appropriate diagnosis.

A $12$-year-old boy with a known history of Ewing sarcoma is brought to the emergency department due to worsening, severe back pain for the past $48$ hours. This morning, he developed weakness in both legs and has been unable to urinate. His temperature is $37.2^\circ\text{C}$ ($99.0^\circ\text{F}$), blood pressure is $112/70\text{ mmHg}$, pulse is $98\text{/min}$, and respirations are $20\text{/min}$. Physical examination reveals $2/5$ motor strength in the bilateral lower extremities, exaggerated deep tendon reflexes at the knees and ankles, and a loss of sensation to pinprick below the level of the umbilicus. Anal sphincter tone is markedly decreased. Arrange the following steps in the most appropriate chronological order for the management of this patient's current condition.

An $8$-year-old girl with a history of homozygous sickle cell anemia (HbSS) is brought to the pediatric emergency department due to a two-day history of progressive shortness of breath, right-sided chest pain, and fever. Her temperature is $38.9^\circ \text{C}$ ($102.0^\circ \text{F}$), blood pressure is $100/60\text{ mmHg}$, heart rate is $128/\text{min}$, and respiratory rate is $34/\text{min}$. Oxygen saturation is $89\%$ on room air. Physical examination reveals shallow breathing and crackles over the right lower posterior lung fields. Laboratory studies show:

* Hemoglobin: $7.1\text{ g/dL}$
* Leukocyte count: $19,500/\mu \text{L}$
* Reticulocytes: $11\%$

Chest radiography demonstrates a new alveolar infiltrate in the right lower lobe. The patient is immediately started on supplemental oxygen, pain control, and intravenous normal saline. Which of the following is the most appropriate additional pharmacologic therapy for this patient?